Kyle, Jenna, and Bryce have been home from Pittsburgh for a week now — what a relief to have that trip over. They spent it at home in Dodgeville with help from Grandma Sue (through Monday morning) and then Grandma Wendy from Monday late afternoon through Thursday midday. Thank goodness for our amazing mothers is all I can say.
They have been adjusting Bryce’s medications since returning, and he has seemed more content the last few days. They were even able to put him in his infant swing for five minutes two times the other day. He also sat on Papa Tom’s lap on Friday for a few minutes, something he hasn’t done since early December.
While Kyle and Jenna expected test results earlier in the week, they learned on Tuesday that they’d be able to talk with Dr. Escolar on Thursday. I’m going to do my best to recap what Jenna relayed to me about all of Bryce’s tests:
- Vision Test: Bryce’s eyesight is still good, and according to the Visual Evoked Potentials test, his brain is correctly interpreting what he’s seeing, which is great news.
- Hearing Test: His hearing is still good, though there is a slight delay in how Bryce is processing what he’s hearing. Dr. Escolar recommended that they just speak in shorter sentences to him and more slowly so he can process their words better.
- Nerve Conduction Test: When a healthy child does this test, their nerve response is typically 100% — meaning they respond immediately. Bryce’s response is about 40%, which Jenna said she felt was a little generous as he is moving his arms and legs very little on his own at this point. Dr. Escolar explained that part of his lack of movement is due to the muscle relaxant medication that he’s on: The medicine relaxes his muscles to a point where even if he wanted to move, it would take him a lot more effort to overcome the effects of it. Why not reduce the amount of medicine, you might be wondering? Without it, Bryce is in pain. It’s a double-edged sword.
- Brain and Lumbar MRI: This test showed that the Krabbe has progressed, meaning that some of the white matter has been effected, and as a result there’s been some slowing of the nerves, but it’s mild at this point.
Dr. Escolar explained that there are four stages to this disease and that Bryce is in stage 2 (there’s often overlap between the stages so how some children progress is different than others):
- Stage 1: Feeding problems; slowing of development/regression
- Stage 2: Tightness and stiffness; extreme irritability; digestive system begins processing more slowly
- Stage 3: Vision begins to go
- Stage 4: Vision and hearing are typically gone; seizures; trouble controlling body temperature
Krabbe is a disease of the brain and peripheral nervous system, and Dr. Escolar was also able to help Kyle and Jenna (and all of us) better understand why certain things are happening with Bryce’s little body. He is constantly clenching his fists, for instance — to the point where he cries when you try to open his hands. She says this is a sign of a “disease of the brain.” His sensitivity to touch and need to be held are signs of a disease of the peripheral nervous system.
Other things that Dr. Escolar told them:
- Most families see the most changes within the first six months of the disease and then it begins to plateau.
- Over the next two months, she expects Bryce to become even tighter and weaker (she wants them working with a physical therapist regularly with the goal of keeping his joints loose and to work on range of motion).
The most important message that she shared with Kyle and Jenna (at least from my perspective), is that with proper care and interventions, and if he stays healthy besides the disease, Bryce could live two to three years.